The overall goal of this Specialized Center of Research in Pediatric Pulmonary Disease is enhanced understanding and improved therapy of infant respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD) by: 1) definition of metabolic regulatory mechanisms controlling the biosynthesis and intracellular transfer of essential pulmonary phospholipids, particularly saturated phosphatidylcholine; 2) elucidation of the role of exogenous and endogenous precursors in lung metabolism; 3) identification of factors influencing the timing of lung maturation, the risk of neonatal RDS, and the susceptibility to BPD in early infancy; 4) delineation of metabolic and nutritional disorders complicating RDS and BPD; and 5) development of therapeutic measures which will correct associated malnutrition and thereby improve the process of recovery. Basic projects will address the following topics: glucoregulation in pulmonary phospholipid metabolism; lung uptake of choline and liposomal phosphatidylcholine; intracellular transfer of phosphatidylcholine in lung; isolation and characterization of fatty acid binding protein from lung; influences on timing of fetal primate lung maturation, including morphologic aspects of lung development concerning alveolar apithelium and neuroendocrine cells; the fetal uptake and pulmonary effects of dexamethasone; development of regulatory peptides in normal and abnormal lung tissue. Methods to be utilized include measurement of phospholipid concentrations and rates of synthesis, determination of enzyme activities, isolation of proteins, evaluation of the kinetics of transfer of substances to the lung, physiologic evaluation of lung surfactant, and morphometric analyses at the light and electron microscopy levels. These methods will be applied to various animal models and lung study systems including Rhesus monkeys (Macacca mulatta), diabetic rats, the isolated/perfused lung, and type II pneumonocytes in culture. Clinical projects will focus on RDS and BPD. One major study will deal with evaluation and treatment of malnutrition in RDS. Clinical Studies will include extensive follow-up and epidemiologic assessment of infants entered into protocols designed to improve the outcome of children managed with intensive care in a perinatal center.